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Mortality in sickle cell disease. Life expectancy and risk factors for early death
Open accessAbstract
BACKGROUND: Information on life expectancy and risk factors for early death among patients with sickle cell disease (sickle cell anemia, sickle cell-hemoglobin C disease, and the sickle cell-beta-thalassemias) is needed to counsel patients, target therapy, and design clinical trials. METHODS: We followed 3764 patients who ranged from birth to 66 years of age at enrollment to determine the life expectancy and calculate the median age at death. In addition, we investigated the circumstances of death for all 209 adult patients who died during the study, and used proportional-hazards regression analysis to identify risk factors for early death among 964 adults with sickle cell anemia who were followed for at least two years. RESULTS: Among children and adults with sickle cell anemia (homozygous for sickle hemoglobin), the median age at death was 42 years for males and 48 years for females. Among those with sickle cell-hemoglobin C disease, the median age at death was 60 years for males and 68 years for females. Among adults with sickle cell disease, 18 percent of the deaths occurred in patients with overt organ failure, predominantly renal. Thirty-three percent were clinically free of organ failure but died during an acute sickle crisis (78 percent had pain, the chest syndrome, or both; 22 percent had stroke). Modeling revealed that in patients with sickle cell anemia, the acute chest syndrome, renal failure, seizures, a base-line white-cell count above 15,000 cells per cubic millimeter, and a low level of fetal hemoglobin were associated with an increased risk of early death. CONCLUSIONS: Fifty percent of patients with sickle cell anemia survived beyond the fifth decade. A large proportion of those who died had no overt chronic organ failure but died during an acute episode of pain, chest syndrome, or stroke. Early mortality was highest among patients whose disease was symptomatic. A high level of fetal hemoglobin predicted improved survival and is probably a reliable childhood forecaster of adult life expectancy.
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“Elevated levels of fetal hemoglobin (consisting of two alpha and two gamma chains) are associated with improved morbidity and mortality in patients with TDT and SCD.[20][21][22]”
From CRISPR-Cas9 Gene Editing for Sickle Cell Disease and β-Thalassemia (2021) · cites this paper as [21]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“the presence of chronic kidney disease is an independent predictor of pulmonary vascular disease and early mortality in adults with SCD (68, 202).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [68]
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“In 3764 patients in the pre-hydroxyurea era, HbF .8.6% was associated with improved survival.60”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [60]
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“Patients affected by sickle cell anemia and β-thalassemia experience a milder course of the disease if they express elevated levels of fetal hemoglobin in adulthood (Platt et al., 1994; Weatherall, 2001).”
From Reactivation of developmentally silenced globin genes by forced chromatin looping (2014) · cites this paper as Platt et al., 1994
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Citations
Cites
Pain in Sickle Cell Disease: Rates and Risk Factors
Platt, O. S., Thorington, B. D., Brambilla, D. J., Milner, P. F., Rosse, W. F., Vichinsky, E., & Kinney, T. R. (1991) · New England Journal of Medicine · doi:10.1056/nejm199107043250103 · Cited by 1,546
Self-citationLEIKIN, S. L., Gallagher, D. M., Kinney, T. R., Sloane, D., Klug, P., & RIDA, W. N. (1989) · PubMed · Cited by 361
Self-citationMortality in Children and Adolescents With Sickle Cell Disease
LEIKIN, S. L., Gallagher, D. M., Kinney, T. R., Sloane, D., Klug, P. P., & RIDA, W. N. (1989) · PEDIATRICS · doi:10.1542/peds.84.3.500 · Cited by 319
Self-citationCauses of death in sickle-cell disease in Jamaica.
Thomas, A. N., Pattison, C. W., & Serjeant, G. R. (1982) · BMJ · doi:10.1136/bmj.285.6342.633 · Cited by 273
Embury, S. H., Miller, J., Dozy, A. M., Kan, Y. W., Chan, V., & Todd, D. (1980) · Journal of Clinical Investigation · doi:10.1172/jci109984 · Cited by 260
α-Globin gene organisation in blacks precludes the severe form of α-thalassaemia
Dozy, A. M., Kan, Y. W., Embury, S. H., Mentzer, W. C., Wang, W. C., Lubin, B. H., DAVIS, J. R., & Koenig, H. M. (1979) · Nature · doi:10.1038/280605a0 · Cited by 221
The Haemolytic Anaemias: Congenital and Acquired
Signy, A. G. (1955) · Journal of Clinical Pathology · doi:10.1136/jcp.8.4.353-a · Cited by 174
Steinberg, M. H., Rosenstock, W., Coleman, M. B., Adams, J. D., Platica, O., Cedeno, M., Rieder, R. F., Wilson, J., Milner, P., & West, S. (1984) · Blood · doi:10.1182/blood.v63.6.1353.1353 · Cited by 161
Self-citationThe variable expression of sickle cell disease is genetically determined.
Powars, D., Chan, L. S., & Schroeder, W. (1990) · PubMed · Cited by 155
The cooperative study of sickle cell disease: review of study design and objectives.
Gaston, M. H., & Rosse, W. F. (1982) · PubMed · Cited by 129
Self-citation
Research works printed but not linked: 4
- Diggs LM. Anatomic lesions in sickle cell disease. In: Abramson H, Bertles JF, Wethers DL, eds. Sickle cell disease: diagnosis, management, education, and research. St. Louis: C.V. Mosby, 1973:189-229.
- Powars D, 1990, Semin Hematol, 27, 360
- Gaston M, 1982, Am J Pediatr Hematol Oncol, 4, 197
- Cole TB, 1986, Am J Dis Child, 140, 1255
Cited by
Sorted by citations
Items 801–900 of 1,015
Kleinman, S. H., & Stassinopoulos, A. (2015) · Transfusion · doi:10.1111/trf.13259 · Cited by 47
The changing face of homozygous sickle cell disease: 102 patients over 60 years
Serjeant, G. R., Serjeant, B. E., Mason, K. P., Hambleton, I. R., Fisher, C., & Higgs, D. R. (2008) · International Journal of Laboratory Hematology · doi:10.1111/j.1751-553x.2008.01089.x · Cited by 47
“Survival in SS disease is greater than previously believed, studies in the US (Platt et al., 1994) estimating a median of 42 years (males) and 48 years (females) with similar figures in Jamaica using a standard statistical adjustment and even higher figures with mortality rate adjustment (Wierenga,…”
ResultHorwitz, M. E., Spasojević, I., Morris, A., Telen, M. J., Essell, J. H., Gasparetto, C., Sullivan, K. M., Long, G. D., Chute, J. P., Chao, N. J. A., et al. (2007) · Transplantation and Cellular Therapy · doi:10.1016/j.bbmt.2007.08.050 · Cited by 47
“Recently described clinical and laboratory characteristics such as pulmonary hypertension, renal insufficiency, and leukocytosis, along with the intensity of hemolysis allow for the identification of SCD patients who are destined to succumb to complications before the median survival of approximately 40-50 years [8-10…”
BackgroundAllogeneic hematopoietic stem cell transplantation in children with sickle cell disease
Locatelli, F., & Pagliara, D. (2012) · Pediatric Blood & Cancer · doi:10.1002/pbc.24177 · Cited by 51
“Over the last three decades, many patients with SCD have benefited from advances in supportive and interventional therapies and this disease, rather than simply being a life-threatening condition in childhood, has become for many patients a chronic illness of adulthood, characterized by a progressive development of si…”
Background2015 Clinical trials update in sickle cell anemia
Archer, N. M., Galactéros, F., & Brugnara, C. (2015) · American Journal of Hematology · doi:10.1002/ajh.24116 · Cited by 49
Established and experimental treatments for sickle cell disease.
Franceschi, L. D., & Corrocher, R. (2004) · PubMed · Cited by 47
Validation of a Low-Cost Paper-Based Screening Test for Sickle Cell Anemia
Piety, N. Z., Yang, X., Kanter, J., Vignes, S. M., George, A., & Shevkoplyas, S. S. (2016) · PLoS ONE · doi:10.1371/journal.pone.0144901 · Cited by 47
“Universal screening of newborns in the United States and many other developed countries using these highly accurate laboratory methods has enabled early intervention and treatment of SCA, which have significantly reduced SCA-related early childhood mortality and have contributed to the overall improvement in the quali…”
BackgroundInfluentialKoehl, B., Nivoit, P., Nemer, W. E., Lenoir, O., Hermand, P., Pereira, C., Brousse, V. A. M., Guyonnet, L., Ghinatti, G., Benkerrou, M., et al. (2017) · Haematologica · doi:10.3324/haematol.2016.156869 · Cited by 51
“studies have highlighted the important role of polymorphonuclear neutrophils (neutrophils), both during an acute VOC(3) and in the associated long-term morbidity and mortality.(4) Interestingly, a high, steady-state, peripheral white cell count is a risk factor for both significant morbidity – stroke, pulmonary compli…”
BackgroundWonkam, A., Mnika, K., Bitoungui, V. J. N., Chemegni, B. C., Chimusa, E. R., Dandara, C., & Kengne, A. P. (2017) · British Journal of Haematology · doi:10.1111/bjh.15011 · Cited by 48
“Frequent VOC were a marker for disease severity and premature mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) (Platt et al, 1991, 1994), and in modern cohorts in the United States of America (USA) (Darbari et al, 2013; Elmariah et al, 2014).”
BackgroundInfluentialChoi, S., O’Neil, S., Joshi, A. A., Li, J., Bush, A., Coates, T. D., Leahy, R. M., & Wood, J. C. (2019) · American Journal of Hematology · doi:10.1002/ajh.25570 · Cited by 51
“Men with SCD have lower life expectancies and higher rates of silent strokes than women with SCD.(83)”
BackgroundThe Influence of Renal Function on Hydroxyurea Pharmacokinetics in Adults With Sickle Cell Disease
Yan, J., Ataga, K. I., Kaul, S., Olson, J. S., Grasela, D. M., Gothelf, S., Kutlar, A., & Orringer, E. P. (2005) · The Journal of Clinical Pharmacology · doi:10.1177/0091270004273526 · Cited by 47
“Furthermore, high levels of Hb F have been reported to be associated with improved survival in these patients.(5)”
BackgroundAdherent leukocytes capture sickle erythrocytes in an in vitro flow model of vaso‐occlusion
Finnegan, E. M., Turhan, A., Golan, D. E., & Barabino, G. A. (2006) · American Journal of Hematology · doi:10.1002/ajh.20819 · Cited by 47
“Elevated leukocyte counts are associated with severity of disease progression [29], the risk of stroke [30], and the risk of early death [3].”
BackgroundHaynes, J., Baliga, B. S., Obiako, B. O., Ofori-Acquah, S. F., & Pace, B. S. (2004) · Blood · doi:10.1182/blood-2003-08-2969 · Cited by 47
“HU is the only drug currently approved for the treatment of SCD that is known to ameliorate its most common symptom, pain 3 and common complications associated with death, including the acute chest syndrome.(32,33) The amelioration of symptoms by HU occurs at least in part through its ability to stimulate Hb F synthes…”
BackgroundAnaesthetic care for sickle cell disease
Frietsch, T., Ewen, I., & Waschke, K. F. (2001) · European Journal of Anaesthesiology · doi:10.1046/j.0265-0215.2000.00804.x · Cited by 47
Stallworth, J. R., Jerrell, J. M., & Tripathi, A. (2010) · American Journal of Hematology · doi:10.1002/ajh.21772 · Cited by 47
Albuminuria correlates with hemolysis and NAG and KIM-1 in patients with sickle cell anemia
Hamideh, D., Raj, V. M. S., Harrington, T. J., Li, H., Margolles, E., Amole, F., Garcia-Buitrago, M. T., Ruiz, P., B, G. Z., & Alvarez, O. A. (2014) · Pediatric Nephrology · doi:10.1007/s00467-014-2821-8 · Cited by 46
Fetal hemoglobin in sickle cell anemia: Bayesian modeling of genetic associations
Paola Sebastiani, B., Wang, L., Nolan, V. G., Melista, E., Ma, Q., Baldwin, C. T., & Steinberg, M. H. (2007) · American Journal of Hematology · doi:10.1002/ajh.21048 · Cited by 46
Self-citationMorphine for the Treatment of Pain in Sickle Cell Disease
Gupta, M., Msambichaka, L. A., Ballas, S. K., & Gupta, K. (2015) · The Scientific World JOURNAL · doi:10.1155/2015/540154 · Cited by 46
Advances in clinical research in sickle cell disease
Hagar, W., & Vichinsky, E. P. (2008) · British Journal of Haematology · doi:10.1111/j.1365-2141.2008.07097.x · Cited by 46
“…better care of splenic sequestration (Kinney et al, 1990), and transfusions for primary stroke prevention (Adams et al, 1998) have increased the average life span of sickle cell patients from <20 years to over 40 years of age (Platt et al, 1994), with every indication that this trend is continuing.”
BackgroundImmune mechanisms involved in sickle cell disease pathogenesis: current knowledge and perspectives
de Azevedo, J. T. C., & Malmegrim, K. C. R. (2020) · Immunology Letters · doi:10.1016/j.imlet.2020.04.012 · Cited by 46
Abboud, M. R., Taylor, E. C., Habib, D. M., Dantzler‐Johnson, T., Jackson, S. M., Xu, F., Laver, J. H., & Ballas, S. K. (2000) · British Journal of Haematology · doi:10.1046/j.1365-2141.2000.02358.x · Cited by 46
Day‐care management of sickle cell painful crisis in Jamaica: a model applicable elsewhere?
Ware, M. A., Hambleton, I. R., Ochaya, I., & Serjeant, G. R. (1999) · British Journal of Haematology · doi:10.1046/j.1365-2141.1999.01160.x · Cited by 46
“In the U.S.A., painful crises account for 79–91% of emergency room visits by sickle cell patients and 59–68% of hospitalizations, with average admission durations of 8–11 d (Yang et al, 1997).”
ResultInfluentialOpportunities to improve outcomes in sickle cell disease.
Mehta, S. R., Afenyi‐Annan, A., Byrns, P. J., & Lottenberg, R. (2006) · PubMed · Cited by 46
Klings, E. S., Christman, B. W., McClung, J. P., Stucchi, A. F., McMahon, L. C., BRAUER, M., & Farber, H. W. (2001) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.164.7.2101020 · Cited by 46
g(HbF): a genetic model of fetal hemoglobin in sickle cell disease
Gardner, K., Fulford, T., Silver, N., Rooks, H., Angelis, N., Allman, M., Nkya, S., Makani, J., Howard, J., Kesse-Adu, R., et al. (2018) · Blood Advances · doi:10.1182/bloodadvances.2017009811 · Cited by 43
“HbF levels affect the severity of SCD; patients with higher levels of HbF have fewer complications and live longer.(1,2) We tested the influence of g(HbF) on hospitalization rate in HbSS/HbSb patients and detected tentative association (N 5 304, b 5 0.”
BackgroundNeuropathic pain in individuals with sickle cell disease
Sharma, D., & Brandow, A. M. (2019) · Neuroscience Letters · doi:10.1016/j.neulet.2019.134445 · Cited by 45
Mechanisms of pain in sickle cell disease
Takaoka, K., Cyril, A. C., Jinesh, S., & Radhakrishnan, R. (2020) · British Journal of Pain · doi:10.1177/2049463720920682 · Cited by 50
Nickel, R. S., Osunkwo, I., Garrett, A. P., Robertson, J. M., Archer, D. R., Promislow, D., Horan, J., Hendrickson, J. E., & Kean, L. S. (2015) · British Journal of Haematology · doi:10.1111/bjh.13326 · Cited by 45
“…prior to CT therapy initiation, as suggested by previous studies that demonstrate lleucocytosis is a risk factor for SCD complications (Castro et al , 1994; Platt et al , 1994; Kinney et al , 1999; Quinn et al , 2008), including stroke (Balkaran et al , 1992), the main indication for CT therapy.”
MethodMechanisms, malfunctions and explanation in medicine
Nervi, M. (2009) · Biology & Philosophy · doi:10.1007/s10539-009-9190-x · Cited by 45
“(homozygous for sickle hemoglobin), the median age at death is 42 years for males and 48 years for females ( Platt et al. 1994 ).”
BackgroundQuality of life of adult patients with sickle cell disease
Mann‐Jiles, V., & Morris, D. L. (2009) · Journal of the American Academy of Nurse Practitioners · doi:10.1111/j.1745-7599.2009.00416.x · Cited by 45
“Carriers of the trait do not have the disease, but some red blood cells may change shape if they are severely deprived of oxygen (Joint Center for Sickle Cell and Thalassemic Disorders, 2002; Platt et al., 1994; Quinn, Rogers, & Buchanan, 2004).”
BackgroundKELLY, M. J., Pennarola, B. W., Rodday, A. M., Parsons, S. K., & on behalf of the Journeys to Recovery Study, T. H. S. (2011) · Pediatric Blood & Cancer · doi:10.1002/pbc.24036 · Cited by 45
“The risk of morbidity and mortality with HSCT must be considered alongside the shortened life expectancy and chronic complications in adulthood without transplant [15,16,36,37].”
BackgroundHemoglobin genetics: recent contributions of GWAS and gene editing
Smith, E. C., & Orkin, S. H. (2016) · Human Molecular Genetics · doi:10.1093/hmg/ddw170 · Cited by 45
“the clinical severity of both disorders (2).”
Microfluidic electrical impedance assessment of red blood cell-mediated microvascular occlusion
Man, Y., Maji, D., An, R., Ahuja, S. P., Little, J. A., Suster, M. A., Mohseni, P., & Gurkan, U. A. (2021) · Lab on a Chip · doi:10.1039/d0lc01133a · Cited by 45
Ketamine administration for acute painful sickle cell crisis: A randomized controlled trial
Alshahrani, M. S. S., Alsulaibikh, A. H., Tahan, M. R. E., Alfaraj, S. Z., Asonto, L. P., Almulhim, A., AlAbbad, M. F., Maghraby, N., Aljumaan, M. A., Aljunaid, T. O., et al. (2021) · Academic Emergency Medicine · doi:10.1111/acem.14382 · Cited by 45
Dampier, C., Telen, M. J., Wun, T., Brown, C., Desai, P. C., Rassi, F. A. E., Fuh, B., Kanter, J., Pastore, Y. D., Rothman, J., et al. (2022) · Blood · doi:10.1182/blood.2022015797 · Cited by 45
Inflammatory targets of therapy in sickle cell disease
Owusu-Ansah, A. T., Ihunnah, C. A., Walker, A. L., & Ofori-Acquah, S. F. (2015) · Translational research · doi:10.1016/j.trsl.2015.07.001 · Cited by 46
“Increased leukocyte count is associated with the development of many severe complications of SCD including acute chest syndrome (ACS) and stroke, and with a higher mortality rate (11).”
BackgroundSubcortical and cerebellar volumetric deficits in paediatric sickle cell anaemia
Kawadler, J. M., Clayden, J. D., Kirkham, F. J., Cox, T. S. C., Saunders, D. E., & Clark, C. A. (2013) · British Journal of Haematology · doi:10.1111/bjh.12496 · Cited by 45
“In homozygous sickle cell anaemia, significant advances in newborn screening and paediatric clinical interventions have enabled the life expectancy of patients with SCA to increase dramatically (Platt et al, 1994).”
BackgroundDisability Among Individuals with Sickle Cell Disease
Swanson, M. E., Grosse, S. D., & Kulkarni, R. (2011) · American Journal of Preventive Medicine · doi:10.1016/j.amepre.2011.09.006 · Cited by 45
Acute chest syndrome in the postoperative sickle cell patient
Delatte, S. J., Hebra, A. V., Tagge, E. P., Jackson, S. M., Jacques, K., & Othersen, H. B. (1999) · Journal of Pediatric Surgery · doi:10.1016/s0022-3468(99)90254-3 · Cited by 45
Setty, Y. B. N., & Stuart, M. J. (2002) · Journal of Laboratory and Clinical Medicine · doi:10.1067/mlc.2002.121200 · Cited by 45
Molecular characterization of β-thalassemia genes in an Argentine population
Roldán, A., Gutiérrez, M., Cygler, A. M., Bonduel, M. M., Sciuccati, G. B., & Torres, A. F. (1997) · American Journal of Hematology · doi:10.1002/(sici)1096-8652(199703)54:3
Genetic Variation and Sickle Cell Disease Severity
Kirkham, J. K., Estepp, J. H., Weiss, M. J., & Rashkin, S. R. (2023) · JAMA Network Open · doi:10.1001/jamanetworkopen.2023.37484 · Cited by 48
Ezenwa, M. O., Molokie, R. E., Wang, Z. J., Yao, Y., Suarez, M. L., Angulo, V., & Wilkie, D. J. (2014) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2013.08.020 · Cited by 44
“001, and average pain intensity (F(2,134)=8.”
BackgroundInfluentialHumbert, O., Peterson, C. W., Norgaard, Z. K., Radtke, S., & Kiem, H. (2017) · Molecular Therapy — Methods & Clinical Development · doi:10.1016/j.omtm.2017.11.005 · Cited by 43
Tran, H., Mittal, A., Sagi, V., Luk, K., Nguyen, A., Gupta, M., Nguyen, J., Lamarre, Y., Lei, J., Guedes, A. G. P., et al. (2019) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2019.00056 · Cited by 44
Morgan, R. A., Unti, M. J., Aleshe, B., Brown, D., Osborne, K. S., Koziol, C., Ayoub, P. G., Smith, O. B., O’Brien, R., Tam, C., et al. (2019) · Molecular Therapy · doi:10.1016/j.ymthe.2019.09.020 · Cited by 46
Kucukal, E., Man, Y., Quinn, E., Tewari, N., An, R., Ilich, A., Key, N. S., Little, J. A., & Gurkan, U. A. (2020) · Blood Advances · doi:10.1182/bloodadvances.2020001656 · Cited by 44
Rees, D. C., Kılınç, Y., Ünal, S., Dampier, C., Pace, B. S., Kaya, B., Trompeter, S., Odame, I., Mahlangu, J. N., Ünal, Ş., et al. (2022) · Blood · doi:10.1182/blood.2021013674 · Cited by 44
Physician‐diagnosed asthma and acute chest syndrome: Associations with NOS Polymorphisms
Duckworth, L. J., Hsu, L. L.-Y., Feng, H., Wang, J., Sylvester, J. E., Kissoon, N. “., Sandler, E. S., & Lima, J. J. (2007) · Pediatric Pulmonology · doi:10.1002/ppul.20582 · Cited by 44
How I manage priapism due to sickle cell disease
Olujohungbe, A., & Burnett, A. L. “. (2013) · British Journal of Haematology · doi:10.1111/bjh.12199 · Cited by 44
“It is a multisystem disorder with protean manifestations and associated with a reduced life expectancy (Platt et al, 1994).”
BackgroundDecreased exhaled nitric oxide in sickle cell disease: Relationship with chronic lung involvement
Girgis, R. E., Qureshi, M. A., Abrams, J., & Swerdlow, P. S. (2003) · American Journal of Hematology · doi:10.1002/ajh.10284 · Cited by 44
“Acute chest syndrome (ACS) is the most common cause of death and a history of ACS markedly increases the risk of early mortality in adults with SCD [1].”
BackgroundLongitudinal analysis of pulmonary function in adults with sickle cell disease
Field, J. J., Glassberg, J., Gilmore, A., Howard, J., Patankar, S., Yan, Y., Davies, S. C., DeBaun, M. R., & Strunk, R. C. (2008) · American Journal of Hematology · doi:10.1002/ajh.21176 · Cited by 44
“Pulmonary disease is a common cause of morbidity and mortality among individuals with sickle cell disease (SCD) [1].”
BackgroundCanalli, A. A., Franco‐Penteado, C. F., Traina, F., Saad, S. T. O., Costa, F. F., & Conran, N. (2007) · European Journal Of Haematology · doi:10.1111/j.1600-0609.2007.00926.x · Cited by 44
“High basal leukocyte numbers are associated with increased morbidity and mortality in patients (1); however, the significance of the leukocyte in SCD pathophysiology is now becoming recognised.”
BackgroundCherry, M. G., Greenhalgh, J., Osipenko, L., Venkatachalam, M., Boland, A., Dundar, Y., Marsh, K., Dickson, R., & Rees, D. C. (2012) · Health Technology Assessment · doi:10.3310/hta16430 · Cited by 44
Review: Management of Painful Vaso-Occlusive Crisis of Sickle-Cell Anemia: Consensus Opinion
Mousa, S. A., Momen, A. A., Alsayegh, F., Jaouni, S. K. A., Nasrullah, Z., Saeed, H. A., Alabdullatif, A., Sayegh, M. A., Zahrani, H. A., Hegazi, M., et al. (2010) · Clinical and Applied Thrombosis/Hemostasis · doi:10.1177/1076029609352661 · Cited by 44
Tanabe, P. J., Artz, N., Courtney, D. M., Martinovich, Z., Weiss, K. B., Zvirbulis, E., & Hafner, J. W. (2010) · Academic Emergency Medicine · doi:10.1111/j.1553-2712.2010.00693.x · Cited by 44
“died during a pain episode and other predictors of death included acute chest syndrome, renal failure, seizures, a white blood cell count greater than 15,000, and a low fetal hemoglobin.(2) These data suggest that careful evaluation for life-threatening comorbidities is warranted.”
BackgroundResultInfluentialMccrae, J. D., & Lumley, M. A. (1998) · Journal of Behavioral Medicine · doi:10.1023/a:1018763404868 · Cited by 46
Perfetti, D. C., Boylan, M. R., Naziri, Q., Khanuja, H. S., & Urban, W. P. (2014) · The Journal of Arthroplasty · doi:10.1016/j.arth.2014.10.035 · Cited by 43
Mechanism-driven phase I translational study of trifluoperazine in adults with sickle cell disease
Molokie, R. E., Wilkie, D. J., Wittert, H., Suarez, M. L., Yao, Y., Zhao, Z., He, Y. Z., & Wang, Z. J. (2013) · European Journal of Pharmacology · doi:10.1016/j.ejphar.2013.10.062 · Cited by 43
“It is now clear that the pain experience of those with SCD is much more complex and frequent than the early studies suggested (Dampier et al., 2002a; Dampier et al., 2002b; Maikler et al., 2001; Platt et al., 1994; Platt et al., 1991; Shapiro et al., 1995).”
BackgroundPatel, N. N., Gonsalves, C. S., Yang, M., Malik, P., & Kalra, V. K. (2008) · Blood · doi:10.1182/blood-2008-07-169821 · Cited by 43
Alcohol Abuse in Sickle Cell Disease: The Pisces Project
Levenson, J. L., McClish, D. K., Dahman, B. A., Penberthy, L. T., Bovbjerg, V. E., Aisiku, I. P., Roseff, S. D., & Smith, W. R. (2007) · American Journal on Addictions · doi:10.1080/10550490701525434 · Cited by 43
Cohen, R. T., DeBaun, M. R., Blinder, M. A., Strunk, R. C., & Field, J. J. (2010) · Blood · doi:10.1182/blood-2010-01-265819 · Cited by 43
Academic attainment findings in children with sickle cell disease.
Epping, A., Myrvik, M. P., Newby, R., Panepinto, J. A., Brandow, A., & Scott, J. (2013) · Journal of School Health · doi:10.1111/josh.12064 · Cited by 44
Nielsen, L., Canouï‐Poitrine, F., Jaïs, J., Dahmane, D., Bartolucci, P., Bentaarit, B., Gellen‐Dautremer, J., Remy, P., Kofman, T., Matignon, M., et al. (2016) · British Journal of Haematology · doi:10.1111/bjh.14040 · Cited by 43
“Sickle cell disease (SCD), one of the most common haemoglobinopathies, is associated with a high frequency of chronic kidney disease (CKD), which constitutes an independent risk factor for death in this population (Saraf et al, 2014; Platt et al, 1994).”
BackgroundSocio-environmental exposures and health outcomes among persons with sickle cell disease
Asnani, M., Madden, J. K., Reid, M. E., Greene, L.-G., & LYEW‐AYEE, P. A. (2017) · PLoS ONE · doi:10.1371/journal.pone.0175260 · Cited by 43
Sins, J. W. R., Mager, D. J., Davis, S. C. A. T., Biemond, B. J., & Fijnvandraat, K. (2017) · Blood Advances · doi:10.1182/bloodadvances.2017007211 · Cited by 43
Hyperfiltration predicts long-term renal outcomes in humanized sickle cell mice
Kasztan, M., Fox, B. M., Lebensburger, J. D., Hyndman, K. A., Speed, J. S., Pollock, J. S., & Pollock, D. M. (2019) · Blood Advances · doi:10.1182/bloodadvances.2018028878 · Cited by 43
Sickle cell disease in Africa: an urgent need for longitudinal cohort studies
Wonkam, A., & Makani, J. (2019) · The Lancet Global Health · doi:10.1016/s2214-109x(19)30364-x · Cited by 45
Drug Therapies for the Management of Sickle Cell Disease
Rai, P., & Ataga, K. I. (2020) · F1000Research · doi:10.12688/f1000research.22433.1 · Cited by 43
Antibiotics for treating acute chest syndrome in people with sickle cell disease
Martí-Carvajal, A. J., Conterno, L. D. O., & Knight‐Madden, J. (2015) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd006110.pub4 · Cited by 42
Pharmacotherapy in sickle cell disease – state of the art and future prospects
Hankins, J. S., & Aygün, B. (2009) · British Journal of Haematology · doi:10.1111/j.1365-2141.2009.07602.x · Cited by 43
“In addition, low Hb F concentration is recognized as a predictor of early mortality among patients with SCD (Platt et al, 1994).”
BackgroundAirway hyperresponsiveness and acute chest syndrome in children with sickle cell anemia
Sylvester, K. P., Patey, R. A., Rafferty, G. F., Rees, D. C., Thein, S. L., & Greenough, A. (2007) · Pediatric Pulmonology · doi:10.1002/ppul.20571 · Cited by 43
“Adults and children with sickle cell anemia (SCA) can suffer devastating pulmonary complications, including the acute chest syndrome (ACS) which is an important predictor for early mortality.(1) Adults who have had an ACS episode have been demonstrated to have significantly higher respiratory resistance than those who…”
BackgroundLoukopoulos, D., Voskaridou, E., Kalotychou, V., Schina, M., Loutradi, A., & Theodoropoulos, I. (2000) · Blood Cells Molecules and Diseases · doi:10.1006/bcmd.2000.0328 · Cited by 43
van Beers, E. J., Tuijn, C. F. J. V., Gillavry, M. R. M., van der Giessen, A., Schnog, J. B., Biemond, B. J., & on behalf of the CURAMA study group (2008) · Haematologica · doi:10.3324/haematol.12152 · Cited by 43
Kokoska, E. R., West, K. W., Carney, D. E., Engum, S. A., Heiny, M. E., & Rescorla, F. J. (2004) · Journal of Pediatric Surgery · doi:10.1016/j.jpedsurg.2004.02.027 · Cited by 43
Antoniani, C., Romano, O., & Miccio, A. (2017) · Stem Cells Translational Medicine · doi:10.1002/sctm.17-0192 · Cited by 50
Crosby, L. E., Barach, I. M., McGrady, M. E., Kalinyak, K. A., Eastin, A. R., & Mitchell, M. J. (2012) · Anemia · doi:10.1155/2012/492428 · Cited by 43
“Hydroxyurea (HU), which is used to increase fetal hemoglobin (Hb F), has been shown to decrease morbidity and mortality in patients with SCD [4].”
BackgroundBase Editing of HBG1 and HBG2 Promoters for Sickle Cell Disease
Gupta, A. O., Sharma, A., Frangoul, H., Kanter, J., Mapara, M. Y., Dalal, J. D., Alavi, A., Jaroscak, J., Ayala, E., DiPersio, J. F., et al. (2026) · New England Journal of Medicine · doi:10.1056/nejmoa2504835 · Cited by 45
Ephraim, R. K. D., Osakunor, D. N. M., Cudjoe, O., Oduro, E. A., Asante-Asamani, L., Mitchell, J., Agbodzakey, H., & Adoba, P. (2015) · BMC Nephrology · doi:10.1186/s12882-015-0072-y · Cited by 40
Pack-Mabien, A. V., & Haynes, J. (2009) · Journal of the American Academy of Nurse Practitioners · doi:10.1111/j.1745-7599.2009.00401.x · Cited by 42
“…pain episodes The frequency of vaso-occlusive pain episodes, which are considered the hallmark of SCD, has been identified as an indicator of clinical severity and has been associated with premature deaths in patients more than 20 years of age (Platt et al., 1991, 1994; Martin & Moore, 1997).”
BackgroundInfluentialMajumdar, S., Robertson, Z. M., Robinson, A., Starnes, S. E., Iyer, R. V., & MEGASON, G. C. (2009) · Bone Marrow Transplantation · doi:10.1038/bmt.2009.244 · Cited by 42
Homocysteine Elevation in Sickle Cell Disease
Lowenthal, E. A., Mayo, M. S., Cornwell, P. E., & Thornley‐Brown, D. (2000) · Journal of the American College of Nutrition · doi:10.1080/07315724.2000.10718958 · Cited by 42
“Ischemic complications are a major cause of morbidity and mortality in patients with sickle cell disease [1].”
BackgroundTransplantation of unrelated placental blood cells in children with high-risk sickle cell disease
Adamkiewicz, T. V., Mehta, P., Boyer, M. W., Kedar, A., Olson, T. A., Olson, E. A., Chiang, K., MAURER, D. H., Mogul, M. J., Wingard, J. R., et al. (2004) · Bone Marrow Transplantation · doi:10.1038/sj.bmt.1704606 · Cited by 42
Increased adhesive properties of eosinophils in sickle cell disease
Canalli, A. A., Conran, N., Fattori, A., Saad, S. T. O., & Costa, F. F. (2004) · Experimental Hematology · doi:10.1016/j.exphem.2004.04.010 · Cited by 42
“The rigidity of these deformed cells contributes to the vascular occlusion that characterizes the acute crises of the disease and are a major cause of morbidity [2,3].”
BackgroundPsychosocial determinants of health care utilization in sickle cell disease patients
Reese, F. L., & Smith, W. R. (1997) · Annals of Behavioral Medicine · doi:10.1007/bf02883334 · Cited by 42
“shorter lifespans than those who have milder genetic variants of the disorder, that is, HbSC and HbSI~ Thallassemia (2, 20 ).”
BackgroundBailey, D. B., Armstrong, F. D., Kemper, A. R., Skinner, D., & Warren, S. F. (2008) · Journal of Pediatric Psychology · doi:10.1093/jpepsy/jsn032 · Cited by 42
“Until the early 1980s, SCD was managed symptomatically, with no known cure.”
BackgroundInfluentialPain, Coping and Health Care Utilization in Younger and Older Adults with Sickle Cell Disease
Sanders, K. A., Labott, S. M., Molokie, R. E., Shelby, S. R., & DeSimone, J. (2010) · Journal of Health Psychology · doi:10.1177/1359105309345554 · Cited by 42
Nagel, R. L., & Steinberg, M. H. (2001) · Fetal and Pediatric Pathology · doi:10.1080/15513810109168606 · Cited by 42
Self-citationTriagem neonatal para hemoglobinopatias no Rio de Janeiro, Brasil
Lobo, C. L. D. C., Bueno, L. M., de Moura, P. G., Ogeda, L. L., de Castilho, S. L., & de Carvalho, S. M. F. (2003) · Revista Panamericana de Salud Pública · doi:10.1590/s1020-49892003000200018 · Cited by 42
Atweh, G. F., DeSimone, J., Saunthararajah, Y., Fathallah, H., Weinberg, R. S., Nagel, R. L., Fabry, M. E., & Adams, R. J. (2003) · Hematology · doi:10.1182/asheducation-2003.1.14 · Cited by 42
Determination of Hemoglobin Saturation in Patients With Acute Sickle Chest Syndrome
Kress, J. P., Pohlman, A. S., & Hall, J. B. (1999) · CHEST Journal · doi:10.1378/chest.115.5.1316 · Cited by 42
Sickle Cell Disease at 100 Years
Orkin, S. H., & Higgs, D. R. (2010) · Science · doi:10.1126/science.1194035 · Cited by 40
Sickle cell disease in California: Sociodemographic predictors of emergency department utilization
Wolfson, J. A., Schrager, S. M., Khanna, R., Coates, T. D., & Kipke, M. D. (2011) · Pediatric Blood & Cancer · doi:10.1002/pbc.22979 · Cited by 41
“Additional influential variables include disease severity [36], urbanicity [12,31], gender [6,48], race, and ethnicity [1,2].”
BackgroundBlood bank management of sickle cell patients at comprehensive sickle cell centers
Afenyi‐Annan, A., Willis, M. S., Konrad, T. R., & Lottenberg, R. (2007) · Transfusion · doi:10.1111/j.1537-2995.2007.01434.x · Cited by 41
Campbell, A., Cui, S., Shi, L., Urbonya, R., Mathias, A., Bradley, K., Bonsu, K. O., Douglas, R., Halford, B., Schmidt, L. A., et al. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1104964108 · Cited by 41
“Indeed, HbF was one of the sole predictors of survival in the Cooperative Study of Sickle Cell Disease cohort study of 3,764 patients (6).”
BackgroundA Paper-Based Test for Screening Newborns for Sickle Cell Disease
Piety, N. Z., George, A., Serrano, S., Lanzi, M. C. R., Patel, P. R., Noli, M. P., Kahan, S., Nirenberg, D., Camanda, J., Airewele, G. E., et al. (2017) · Scientific Reports · doi:10.1038/srep45488 · Cited by 41
MicroRNAs and JAK/STAT3 signaling: A new promising therapeutic axis in blood cancers
Sajjadi-Dokht, M., Mohammad, T. A. M., Rahman, H. S., Maashi, M. S., Danshina, S. D., Shomali, N., Solali, S., Marofi, F., Zeinalzadeh, E., Akbari, M., et al. (2021) · Genes & Diseases · doi:10.1016/j.gendis.2021.10.009 · Cited by 41
“Indeed, the most effective treatment for the cycle cell anemia is increasing Hb F production with the formula a2g2 (a combination of two alpha chains and two gamma chains globulin) that improves clinical symptoms and consequently prolongs the patient overall survival rates.(130,131) The therapeutic role of Hb F is rel…”
BackgroundDugdale, M. (2001) · Obstetrics and Gynecology Clinics of North America · doi:10.1016/s0889-8545(05)70206-0 · Cited by 41
Ellison, A. M., & Bauchner, H. (2007) · PubMed Central · Cited by 41
Pells, J. J., Presnell, K. E., Edwards, C. L., Wood, M. E., Harrison, M. O., DeCastro, L. M., Johnson, S., Feliu, M., Canada, S. A., Jonassaint, J., et al. (2005) · PubMed Central · Cited by 41
Metadata
- Authors
- Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P.
- Affiliations
- Boston Children's Hospital (US)
- New England Research Institutes (US)
- Duke University (US)
- Augusta University (US)
- Howard University (US)
- University of Mississippi Medical Center (US)
- Texas Tech University (US)
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1994-06-09
- Volume
- 330
- Issue
- 23
- Pages
- 1639-1644
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2334263927
- Semantic Scholar
- 6bcc7a4d436214c41a4d128f9d221e950eb4962b
- Cited by
- 3,320
- References
- 19
- Citations by year
- 2026: 83 · 2025: 104 · 2024: 123 · 2023: 135 · 2022: 128 · 2021: 167 · 2020: 147 · 2019: 168 · 2018: 140 · 2017: 159 · 2016: 173 · 2015: 165 · 2014: 154 · 2013: 139 · 2012: 115
- FWCI
- 24.43
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Digital Howard (Howard University) (submittedVersion)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Malaria Research and Control
- Health Sciences › Medicine › Hematology › Blood groups and transfusion
- Keywords
- sickle cell disease, sickle cell-hemoglobin C disease, sickle beta-thalassemia, life expectancy, mortality risk factors, acute chest syndrome, vaso-occlusive crisis, stroke, renal failure, seizures, fetal hemoglobin, white blood cell count, organ failure
- MeSH terms
- Adult, Aged, Anemia, Sickle Cell, Cause of Death, Child, Female, Hemoglobin SC Disease, Humans, Life Expectancy, Male, Middle Aged, Probability, Regression Analysis, Risk Factors, Survival Analysis, beta-Thalassemia
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